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HSF Welcome Letter
New society affiliation
It is with great pleasure we can announce that The Hidradenitis Suppurativa Fou...
Abstracts from ‘‘Directions 2006: The First
International Hidradenitis Suppurativa
Research Symposium’’
Dessau, Germany – ...
Administration of ganciclovir to K15-TK mice caused their
death due to gastrointestinal injury. We then treated immuno-
de...
of 4:1. There is an apparent strong influence of sex hormones on
HS/AI and association with acne vulgaris and hirsutism. T...
Conclusion: Surgery is not the only treatment for HS; we need
outcome measures that are more adapted to medical treatment,...
develop and gain approval for a 20 patient, 6-month
investigator-initiated study using another anti-tumor necrosis
alpha a...
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2006 Experimental Dermatology HSF Welcome/Symposium Abstracts

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2006 Experimental Dermatology HSF Welcome/Symposium Abstracts

  1. 1. HSF Welcome Letter New society affiliation It is with great pleasure we can announce that The Hidradenitis Suppurativa Foundation, Inc. (HSF) and Experimental Dermatology (EXD) have agreed on a partnership making EXD the official journal of the HSF. The Editors and the Publisher look forward to working closer with the HSF and wish them welcome aboard. Thomas A. Luger Editor Angela M. Christiano Editor Thomas Trier-Mork Journal Publishing Manager Blackwell Munksgaard Experimental Dermatology is the official journal of the Hidradenitis Suppurativa Foundation, Inc. Michelle Barlow, Robert Howes, Ralf Paus, Christos C. Zouboulis Co-founders of the Hidradenitis Suppurativa Foundation, Inc., San Diego, California, United States. E-mail: info@hs- foundation.org. The Hidradenitis Suppurativa Foundation, Inc. (HSF), a non-profit organization based in San Diego, California, warmly welcomes Experimental Dermatology (EXD) and Blackwell Munksgaard to an exciting alliance that provides the HSF a home in Europe’s leading basic skin research journal. As the official journal of the HSF, EXD joins the HSF and its international medical and scientific advisory board in our global mission to improve the quality of life and quality of care for individuals and families affected by Hidradenitis Suppurativa (HS), also called Acne Inversa. Forging productive and cooperative research efforts, an expanding international network of research- ers met in Dessau, Germany for ‘Directions 2006’, the First International HS Research Symposium, from 30 March 2006 to 2 April 2006. This event was organized by the HSF, the Dessau Medical Center and the EADV Task Force on ‘Acne and Rosacea’. The HSF thanks EXD for the publication of its symposium abstracts which will help provide essential, up-to-date public health information and detail new concepts and research findings on HS pathogenesis and management. Experimental Dermatology 2006: 15: 405 Copyright # 2006 The Authors. Journal compilation # 2006 Blackwell Munksgaard Blackwell Munksgaard . Printed in Singapore EXPERIMENTAL DERMATOLOGY ISSN 0906-6705 405
  2. 2. Abstracts from ‘‘Directions 2006: The First International Hidradenitis Suppurativa Research Symposium’’ Dessau, Germany – March 30–April 2, 2006 Organized by the Hidradenitis Suppurativa Foundation Inc. (HSF), San Diego, CA, USA, www.hs-foundation.org Hidradenitis suppurativa/acne inversa: the clinical challenge Christos C. Zouboulis* Departments of Dermatology and Immunology, Dessau Medical Center, Dessau, Germany; Laboratory of Biogerontology, Dermato-Pharmacology and Dermato-Endocrinology, Institute of Clinical Pharmacology and Toxicology, Charite´ Universitaetsmedizin Berlin, Campus Benjamin Franklin, Berlin, Germany Hidradenitis suppurativa (HS)/acne inversa (AI) is a chronic, recurrent, debilitating skin disease that presents with painful, inflamed lesions in the apocrine gland-bearing areas of the body, most commonly the axillary, inguinal, and anogenital areas. The disorder is associated with several clinical disputes, such as the discussion on the exact skin appendage involved, namely the sweat glands, the apocrine glands or the terminal hair follicles, and the discussion on the pathogenetic background, namely a genetic, hormonal, bacterial, genuine inflammatory, environmental, or physical one. The significance of gender, body mass, and smoking on disease prevalence is still under investigation. Moreover, the major challenge is the choice of the optimal treatment: antibiotics, retinoids, corticosteroids, incision and drainage, local wound care, limited or radical local excision, radiation, laser therapy, and modern drugs, such as biologics, have been proposed. The psychological impact on the patient can be great, encompassing social, personal, and occupational challenges. The sequelae of the disease, including dermal contraction, keloid formation, restricted limb mobility, lymphedema, and fistula formation dominate the long-term burden associated with patient’s quality of life. Significant research is required to elucidate the etiology of HS/AI, classify the clinical picture, identify appropriate drug candidates, and evaluate the social, economic, and psychological impact of the disease. Open questions in hidradenitis suppurativa research: a hair biologist’s perspective Ralf Paus* Department of Dermatology, University Hospital Schleswig- Holstein, University of Luebeck, Luebeck, Germany To clarify the obscure cause(s) of hidradenitis suppurativa (HS), plausible, comprehensive, and testable hypothetical pathogenesis scenarios urgently need to be developed. HS is a chronic inflammatory disorder of the pilosebaceous unit, which occurs in intertriginous, ‘apocrine’ skin after puberty, and in more women than men. The clinical picture is dominated by late events resulting from follicular occlusion and its tissue-destructive, inflammatory sequelae, aggravated by bacterial superinfection. Likely, HS originates from events in the hair follicle epithelium outer root sheath (ORS) rather than the apocrine gland and/or its duct. Genetic and endocrine factors influence HS incidence and course, at least in defined subpopulations. If follicular hyperkeratosis, leading to the retention of follicular material, represents the earliest histological abnormality, while inflammation and apocrine involvement are secondary ones, then the cause of hyperkeratosis and hyperproliferation of ORS keratinocytes mustbeidentified.Innormalskinepithelium,thistypicallyresultsfrom inflammatory stimuli. Because the skin epithelium is a crucial element in the control of innate immunity, one may speculate that the primary defect in HS is an insufficiently controlled and/or abnormal release of pro-inflammatory ‘danger’ signals from the follicle ORS (e.g. chemokines, defensins, TLR ligands). This may only lead to HS, if additional predisposing factors (e.g. genetically lowered threshold for activation, or defective suppression of, innate immune response loops; constitutive overexpression of TLRs or underexpression of immunoinhibitory signals) are present and once pro-inflammatory stimuli are further up-regulated, e.g. by microtrauma, (neuro-) endocrine and/or local microbial signals. In this scenario, hyperkeratosis/hyperproliferation of ORS keratinocytes only represents the first visible sign of a primary imbalance of the intrafollicular controls of epithelial innate immune mechanisms. This chiefly calls for systematic exploration of the hair follicle innate immune system in as yet uninvolved intertriginous skin of HS patients versus normal controls as a key to unravelling the elusive pathogenesis of HS. Role of bulge cells in wound healing: possible implications for hidradenitis suppurativa M. Ito, Y. Liu, Z. Yang, J. Nguyen, R. Morris, F. Liang and G. Cotsarelis* M8 Stellar-Chance Laboratories, University of Pennsylvania School of Medicine, Philadelphia, PA, USA The hair follicle bulge harbors a cluster of quiescent epithelial stem cells, which generate the new lower hair follicle during follicle cycling. The role of bulge cells for maintenance of the hair follicle and epidermis during normal homeostasis and after wounding remains controversial. To address these questions, we targeted the suicide gene, thymidine kinase (TK), or CrePR recombinase to hair follicle bulge cells using a K15 promoter.*Presenting author. Experimental Dermatology 2006: 15: 478–482 Copyright # 2006 The Authors. Journal compilation # 2006 Blackwell Munksgaard Blackwell Munksgaard . Printed in Singapore EXPERIMENTAL DERMATOLOGY ISSN 0906-6705 478
  3. 3. Administration of ganciclovir to K15-TK mice caused their death due to gastrointestinal injury. We then treated immuno- deficient mice carrying K15-TK skin grafts with ganciclovir. This resulted in injury of bulge cells within several days. Loss of hair and permanent injury to hair follicles ensued. Dermal scarring was also present. The epidermis without follicles survived for several months. To further assess the contribution of bulge cells to epidermis, we used double transgenic K15-crePR1, R26R mice. After 5 days of treatment with RU-486 (time 0), bulge cells and a small fraction of epidermal cells expressed LacZ under control of the ROSA promoter. To determine whether bulge cells moved into the epidermis under normal homeostatic conditions, we counted labeled epidermal basal cells at times 0, 30 days, and 180 days. We found that the percentage of labeled epidermal basal cells (approximately 1%) remained constant, indicating an absence of cell movement from the bulge to the epidermis. After punch wounding, bulge progeny were detected in a radial pattern in the re-epithelialized area. Radial streaks of bulge cell progeny emanated from the hair follicles at the wound edge. Bulge cell progeny proliferated and expressed normal epidermal differentiation markers such as keratin 10 and loricrin. Overall, these results indicate that follicular stem cells in the bulge are necessary for hair follicle survival and that these cells functionally contribute to epidermal regeneration in response to wounding; however, the epidermis self-renews autonomously of the bulge under normal conditions. The role of bulge cells in hidradenitis suppurativa (HS) is not known; however, pathological studies suggest aberrant proliferation of the hair follicle may accompany HS and inappropriate stem cell activation and differentiation could be a component of this disorder. The K15-crePR1 transgenic mouse will serve as a powerful tool for evaluating the role of hair follicle stem cells in mouse models of HS. Studying hair follicle stem cells with recently defined markers for these cells will be useful for evaluating stem cell behavior in HS lesions. Mapping of two genetic loci for autosomal dominant hidradenitis suppurativa W. H. Irwin McLean1 *, Pam Wood1 , Alan D. Irvine2 and Jan von der Werth3 1 Human Genetics Unit, Division of Pathology and Neuroscience, Ninewells Hospital and Medical School, University of Dundee, Dundee, UK; 2 Department of Paediatric Dermatology, Our Lady’s Hospital for Sick Children, Crumlin, Dublin, Ireland; 3 Department of Dermatology, Conquest Hospital, Hastings, UK Hidradenitis suppurativa (HS) is a severe and debilitating skin condition that is relatively common in the population. In many cases, there is a strong family history showing autosomal dominant inheritance with incomplete penetrance. We identified several large kindreds from the United Kingdom and Ireland showing dominant transmission and carried out two genome-wide scans by genetic linkage analysis with 400 closely spaced microsatellite markers. This resulted in the identification of two separate genetic loci that show statistically significant linkage with HS. Marker D19S414 gave a significant log-of-the-odds (LOD) score of 3.66 in a single kindred and represents robust genetic linkage. Recombination with markers D19S911 and D19S1170 limited the interval to a 16.5-Mb region on chromosome 19, containing at least 35 known or strongly predicted genes. Many of these are transcription factors thought to be involved in modulation of the immune system, which may be consistent with HS. Two additional kindreds did not map to this locus but instead showed linkage to marker D6S290, giving a maximum combined 2-point LOD score of 4.0 with no recombination. Visible recombinants narrowed this disease interval to a 1.48-Mb region between D6S440 and D6S441 on chromosome 6q25.1-25.2 containing six genes. We have already screened a large number of exons in both loci for mutations without identifying any obvious mutations. The eventual identification of genes causing HS will shed light on the pathomechanisms underlying inherited forms of the disease and, ultimately, will set the scene for rational design of therapies aimed at treating the root causes of this disorder. Genetic analysis of three large Indian pedigrees with autosomal dominant hidradenitis suppurativa Uppala Radhakrishna1,2 *, Timir Y. Mehta3 , Jitendra V. Solanki4 and Swapan K. Nath5 1 Department of Genetics, Boystown National Research Hospital, Omaha, NE, USA; 2 Green Cross Blood Bank & Genetics Research Centre, Ahmedabad, Gujarat, India; 3 Samarpan Medical & Research Organization, Modasa, Gujarat, India; 4 Department of Animal Genetics & Breeding, Veterinary College, Gujarat Agriculture University, Anand, Gujarat, India; 5 Arthritis and Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA Hidradenitis suppurativa (HS: OMIM 142690) is an aggravating chronic inflammatory skin condition characterized by swollen, painful, inflamed lesions in the axillae, groin, and other parts of the body that contain apocrine glands. Incidence ranges from 0.1/100 to 4/100 in adults. It affects more females than males. The risk of developing non-melanoma skin cancer is very high among patients with HS (Arch Dermatol 2001: 137: 730–734). This chronic condition is familial, which supports its genetic basis. However the causative gene(s) is not yet identified. We have studied three large Indian hidradenitis suppurativa families with an apparent autosomal dominant mode of inheritance and 100% penetrance. No skipping of generations was observed. Pedigrees consist of 149 individuals, including 48 affecteds (20 males/28 females). The age of onset is 25–35 years. Histopathological studies were conducted in selected affecteds. The expression of the phenotype was quite variable within these families, and the majority was severely affected. All affecteds had typical characteristics of HS. Clinical findings included folliculitis, gastrointesinal (GI) polyps, sinuses axillae, cutaneous scars, retinal haemorrhage, epidermoid carcinoma, and polymorph function defects. Six of the affected females had hirsutism. Affected body parts included tops of inner thighs, genitals, groin, buttocks, and under the breasts in women. Skin grafting was performed in some of the affecteds. A few deaths due to squamous cell carcinoma were reported in these families. Cytogenetic analysis of two affecteds from each family did not show any abnormality. Genomewide linkage analysis is in progress to map the elusive locus and provide a target for positional cloning. A DNA array project for hidradenitis suppurativa/acne inversa Sabine Fimmel1 *, Evgenia Makrantonaki1,2 and Christos C. Zouboulis1,2 1 Laboratory of Biogerontology, Dermato-Pharmacology and Dermato-Endocrinology, Institute of Clinical Pharmacology and Toxicology, Charite´ Universitaetsmedizin Berlin, Campus Benjamin Franklin, Berlin; 2 Departments of Dermatology and Immunology, Dessau Medical Center, Dessau, Germany Hidradenitis suppurativa (HS)/acne inversa (AI) is a chronic skin disorder involving the apocrine glandular zones, where inflammation of sebaceous glands and terminal hair follicles can be identified. The exact aetiology of HS/AI is unclear. The reported incidence is 1:300 with a female-to-male predominance HSF Abstracts 479
  4. 4. of 4:1. There is an apparent strong influence of sex hormones on HS/AI and association with acne vulgaris and hirsutism. The disease occurs at post puberty and disease activity declines at the climacteric, whereas improvement is seen during pregnancy. Most HS/AI patients have normal androgen profiles and apocrine glands, nevertheless, there have been reports of symptomatic improvement with the use of anti-androgen therapy. This DNA array project studies differences in gene expression of axillary apocrine secretory cells in vivo, between HS/AI patients and controls. Additionally, the gene expression pattern in axillary apocrine secretory cells of age-matched men and pre- vs. post- menopausal women is evaluated to identify those genes, whose expression is linked to circulating androgen levels, and highlights any gender/age differences in apocrine gland function. These experiments will further identify genes and biochemical pathways that could act as new targets for classical drugs and also targets for siRNA molecules in the treatment of HS/AI. Finding models for skin diseases: successes and failures John P. Sundberg1,2 * and Lloyd E. King Jr2 1 The Jackson Laboratory, Bar Harbor, ME, USA; 2 Vanderbilt University, Nashville, TN, USA Hidradenitis suppurativa (HS) is a disfiguring and debilitating, relatively rare disease in humans. Reports of similar diseases in domestic animals are also very rare. A clinically similar disease was reported in dogs, particularly in Collies and Shelties, but this was later re-evaluated and determined to be a form of vesicular cutaneous lupus erythematosus. The alternative term for HS, acne inversa, suggests forms of acne in domestic animals might provide an alternative approach to finding a model. Mice, unlike dogs and cats, do not generally develop acne. However, the icthyosis mutation (Lbric ) does develop bacterial infections within the infundibulum that may result in furunculosis. If combined with various immunodeficiencies, through the creation of double mutant mice, then this might provide a useful model. However, finding spontaneous or genetically engineered mouse models usually result in obtaining the best tools. The success obtained by developing or finding simple single gene mutation or complex polygenic disease models for pseudoxanthoma elasticum and alopecia areata, respectively, through support from their respective foundations, provide a road map on how such models can be found, defined, and provide unique tools to unravel the complex mechanisms of HS and, more importantly, provide preclinical tools for drug efficacy testing approaches. Cytokeratin expression in hidradenitis suppurativa and pilonidal sinus Ichiro Kurokawa1 *, Arata Hakamada1 , Ken-ichi Isoda1 , Kei-ichi Yamanaka1 , Hitoshi Mizutani1 and Airo Tsubura2 1 Department of Dermatology, Mie University Graduate School of Medicine, Edobashi, Tsu, Mie, Japan; 2 Department of Pathology, Kansai Medical University, Fumizono-cho, Moriguchi, Osaka, Japan Purpose: The aim of our study was to elucidate the pathogenesis of hidradenitis suppurativa (HS) and pilonidal sinus (PS) by evaluating their cytokeratin (CK) expression. Methods: CK expression in HS (n ¼ 15) and PS (n ¼ 9) was studied immunohistochemically using six anti-keratin antibodies. Results: In HS and PS, three types of epithelia were identified: infundibular-like keratinized epithelium (type A), non-infundibular keratinized epithelium (type B), and non- keratinized epithelium (type C). Type A contained CK1, 10, and 14 similar to normal infundibulum, but it did not contain CK17. In types B and C, CK14, 16, and 17 were detected similar to that in normal outer root sheath. In tumor nests of squamous cell carcinoma derived from HS, CK 14, 17, and 19 were detected. Conclusions: Based on CK expression in HS and PS, sinus epithelium in HS and PS may possess characteristics of fragility, hyperproliferation, and undifferentiation. The potential role of acetylcholine receptors in acne inversa (HS) pathogenesis Hjalmar Kurzen*, Anisa Hana, Giovanni Frongia, Sergij Goerdt and Alexei Gratchev Department of Dermatology, University Medical Center Mannheim, University of Heidelberg, Heidelberg, Germany Acne inversa is a chronic inflammatory disorder that has been shown to be influenced by tobacco smoking, which may represent a ‘natural’ model of exogenous nicotine-mediated activation of the nicotinic acetylcholine receptors (AChR). In previous works, we have provided a concise mapping of AChR present in normal skin and have demonstrated a crucial role of AChR in terminal differentiation and barrier formation. In addition, several studies suggest a role for the non-neuronal cholinergic system in immunomodulation. To date, the AChR composition of the cells involved in the pathogenesis of acne inversa has not yet been characterized. Using immunohistochemistry and RT – PCR, we could show that nicotinic a3, a5, a7 and a9 AChR as well as muscarinic M1 – M5 AChR are produced in lesional epidermis in a pattern comparable with normal epidermis. In vitro and in lesional skin, we could demonstrate the presence of a3, a5 and a7 nAChR as well as M1 –M5 mAChR in variable amounts on macrophages. In lesional epidermis, the choline-acetyltransferase (ChAT)- reactivity was particularly pronounced in the epidermal basal layer, while in the sinus tracts, ChAT reactivity was extended to all epithelial layers. High levels of ChAT especially in the hair follicle infundibulum indicate that endogenously produced ACh may act synergistically with tobacco-delivered nicotine in aggravating infundibular hyperkeratosis. ChAT could not be detected in macrophages in vitro or in vivo indicating that macrophages do not actively contribute to ACh signalling in acne inversa but may rather be targets of ACh, and hence nicotine induced immunomodulation. Clinical characteristics and outcome measures in hidradenitis suppurativa research Jean Revuz* Department of Dermatology Hoˆpital Henri-Mondor, Paris XII University, Cre´teil, France Introduction: Outcome measures for medical treatment of HS are needed. Patients and methods: One hundred and sixty-four consecutive patients were prospectively evaluated. A new score was evaluated: HSPAIN ¼ pain intensity on a visual analog scale (VAS 1-10) Â number of painful days/month. Results: Mean age was 34 years (+ 11), sex ratio F/M 2.8:1. Forty-two per cent were over weighted; 82% were smokers, 30% had a family history of HS. Age at onset was 23 years. Hurley’s class was I ¼ 72%; II ¼ 25 %; III ¼ 3%. Mean Sartorius’ score (1) was 22 (range: 3–72). Pain on VAS was 4.1 for lower localizations, and its duration was >15 days/ month for 25% of patients. Patients with a Sartorius’ score > median were older (P ¼ 0.003), more frequently overweighted (0.02). The mean HSPAIN scored 7.9 (0–30). It was highly correlated to Sartorius’ score (P < 10-3). This new score has been used in therapeutic trials of dalacine – rifadin combination and of zinc gluconate. Quality of life study of 61 patients emphasizes the strong impact of HS compared with other dermatological diseases; patients with an early onset, long duration and continuous evolution are particularly affected. HSF Abstracts 480
  5. 5. Conclusion: Surgery is not the only treatment for HS; we need outcome measures that are more adapted to medical treatment, i.e. integrating pain which is the major burden of this disease. Reference: 1. Sartorius K, Lapins J, Emtestam L, Jemec GB. Suggestions for uniform outcome variables when reporting treatment effects in hidradenitis suppurativa. Br J Dermatol: 2003: 149: 193–227. Surgical treatment options for hidradenitis suppurativa and critical review of own experience Wolfgang Christian Marsch* Universitatsklinik und Poliklinik fur Dermatologie und Venerologie, Martin-Luther-Universitat Halle-Wittenberg, Halle, Saale, Germany HS (acne inversa) is a chronic, progressive, initially inflammatory, ultimately a fistulating and scarring disease affecting apocrine gland-bearing skin areas. Late phases afford a broad surgical removal of affected skin areas including subcutaneous fatty tissue, with secondary mesh grafting after a period of granulation tissue formation. Fifty-three patients have been treated surgically at our Dermatology Department. Long-term results are excellent concerning satisfaction of the patients and functional objectives. Local recurrences or development of new lesions in formerly unaffected areas were noticed only in some patients who did not stop smoking. Patient details were as follows: gender distribution: male (M) 20 (38%), female (F) 33 (62%), age: M 19–62 (average 40.7), F 15–56 (average 35.4), onset: M 16–57 (32.2), F 8–50 (25.5), duration: 3 months to 37 years (8.0), F 6 months to 37 years (9.9). Sites mainly affected: axillary and perigenital. Specific regions for men: perineum and rima ani, for women: inguinal, submammary and abdominal. Multiple anatomical regions involved: men 40%, women 91%. Familiarity 0.4%. Associated acne papulo-pustulosa or nodulo-cystica (¼conglobata): 19%. Cigarette smokers: men 100%, women 67%. Excised material from each operation was carefully examined histologically. The results endorse the concept of ‘acne inversa’ by recognizing a perifollicular accumulation of lymphocytes simultaneously at different infrainfundibula of terminal hair follicles. However, a follicular hyperkeratosis seems secondary to this, follicular perforation, and a combination of sinus, abscess and scar formation are most obviously tertiary events. Therefore, HS seems to be an inflammatory, probably an immunological disease with an initially strictly dermal target, even followed by an intradermal horizontal propagation. Laser flux imaging could visualize the subclinical peripheral extension of the basically dermal perifollicular inflammation. Biologics may have a beneficial effect on these early or perpetuating inflammatory events; however, thus far surgery remains the first-line therapy in late phases of the disease. Surgical treatment options for hidradenitis suppurativa and critical review of own experience† Anirban Mandal* and J. Watson Department of Plastic and Reconstructive Surgery, St. John’s Hospital, Livingston, Scotland Background: Hidradenitis suppurativa has always been a challenging area to the plastic and reconstructive surgeon. The aetiology is idiopathic, and medical treatment offers temporary relief only. Radical surgical excision, therefore, is the mainstay of active management. Aim: To present and evaluate a series of 106 patients of hidradenitis suppurativa treated in the regional plastic surgery unit of southeast Scotland between January 1990 and December 2000. Patients: Median age at onset was 36 years (range 17– 70 years). The patients were predominantly females (88.78%) and heavy smokers (98.1%). Median age before active surgical intervention was six years (range 1–30 years). The patients were divided into a mild group (n ¼ 64) and a severe group (n ¼ 42). All patients had broad-spectrum antibiotics and multiple incision and drainage procedures before referral from general practitioners, dermatologists or general surgeons. One hundred sites were managed by primary closure (mostly in the ‘mild’ group); 29 resurfaced with split skin grafts and 14 with local, fasciocutaneous or musculocutaneous flaps (mostly in the ‘severe’ group). Median postoperative follow-up period was 3 years (range 1–7 years). Results: In the ‘primary closure’ series, recurrence rate requiring at least one secondary operation was 69.88%. There was no recurrence, no serious complications and no revision operations in the ‘graft’ and the ‘flap’ series. Conclusion: A high index of suspicion is required before contemplating primary closure in hidradenitis suppurativa, even in the ‘mild’ variety. This condition should always be treated aggressively by radical excision of all hair-bearing areas and reconstructed with a graft or a flap. Surgical treatment of acne inversa (hidradenitis suppurativa): a 20-year experience Wolfgang Hartschuh* Department of Dermatology, University of Heidelberg, Heidelberg, Germany Acne inversa (AI) is caused by follicular hyperkeratosis in intertriginous areas rich in apocrine glands followed by occlusion and rupture of the follicle and inflammation. Sinus tracts, scarring and often contraction with limitation in mobility may occur. There is a world-wide consensus that in chronic disease surgical removal of all involved tissue as early as possible is the most effective treatment modality. The aim of this study is to demonstrate our operative strategy, including postoperative wound care and prevention, the results and pitfalls in the treatment of AI. The operations are increasingly performed in tumescence anaesthesia, followed by secondary healing. Only removal of extended skin areas in the inguino-genital and ano-perineal regions demand general anaesthesia. In axillary regions, all hair-bearing skin including the sweat glands is removed to obtain a hair-free, dry skin area. In the other regions with ill- defined hair and apocrine gland areas, only involved indurated skin is excised. For early limited disease with fluctuant abscesses, unroofing instead of mere incision and drainage is a good first option. Professional wound care with shaving and stretching of the wound margins is very important to avoid premature wound closure. Locally applied disinfectant soaps and 2% triclosane ointments are effective in pre- and postoperative skin care. Follow-up evaluation and collaboration among surgeons and dermatologists and an improved insight in the pathogenesis of AI are mandatory for the successful long- term management of patients afflicted with this complex and debilitating disease. Biologics in the treatment of hidradenitis suppurativa: etanercept (preliminary report on clinical trial) and infliximab (case study) David Richard Adams* Penn State Hershey Medical Center, Hershey, PA, USA A teenager with significant hidradenitis suppurativa (HS) and ulcerative colitis was treated with infliximab infusion. The response was rapid and dramatic. This response led us to †Abstract edited from: Experience with different treatment modules in hidradenitis suppurativa: a study of 106 cases. Surgeon 2005: 3: 23–26. HSF Abstracts 481
  6. 6. develop and gain approval for a 20 patient, 6-month investigator-initiated study using another anti-tumor necrosis alpha agent, etanercept. The study is double-blinded and placebo controlled using etanercept 50 mg subcutaneous injection twice weekly. For the first 3 months, one-half of the patients will receive placebo and other half active drug. Neither patient nor investigator will know whether active drug or placebo is used. In the last 3 months, all patients will receive active drug. The purpose of the study is to evaluate etanercept effectiveness and adverse effects when used for HS. The study is still in progress, but preliminary results are encouraging. Infliximab for hidradenitis suppurativa: report of seven consecutive patients Alain Dupuy*, Laurence Fardet, Delphine Kerob, Annabelle Levy, Herve´ Bachelez, Patrice Morel and Celeste Lebbe Department of Dermatology, Hopital Saint-Louis, Paris, France Purpose: To evaluate all consecutive patients treated with infliximab for hidradenitis suppurativa (HS). Patients and methods: Within 1 year, all consecutive patients seen in our department for HS (1) resistant to usual medical therapies (2) which could not be easily cured by surgery (3) not treated with new medication within 2 months before inclusion were treated intravenously with infliximab (5 mg/kg) without corticosteroids premedication. Four infusions were planned (week 0, week 2, week 6 and week 10) before the interruption of therapy and follow-up. Clinical activity of HS and quality of life of the patients were assessed immediately before the first, the third and the fourth infusions of infliximab. Results: Seven patients were included. Five completed the four infusions. Two patients received only three infusions because of severe side effects. The Sartorius score moderately improved with infliximab (mean score at week 0: 94 + 39, at week 6: 71 + 38 and at week 10: 83 + 48). At week 6, patients judged the efficacy of therapy as marked (n ¼ 1), moderate (n ¼ 4) or null (n ¼ 2). At week 10, five patients were evaluated and judged this efficacy as marked (n ¼ 2), moderate (n ¼ 2) or null (n ¼ 1). The mean Skindex-29 score varied from 22 + 11 (E: 25 + 9, S: 13 + 5, F: 28 + 12) at week 0 to 18 + 10 (E: 22 + 8, S: 12 + 8, F: 22 + 12) at week 10. Conclusion: The efficacy of infliximab in severe HS is partial. More experience is needed before finding a place for infliximab in the therapeutic armamentarium for HS. The role of stem cells in suppurative environments Dolores Herreros*, Mariano Garcı´a-Arranz, Isabel Pascual and Damia´n Garcı´a-Olmo Cathedra UAM-CELLERIX for Cell Therapy and Regenerative Medicine, La Paz University Hospital and Universidad Auto´noma de Madrid, Madrid, Spain Purpose: The management of suppurative perianal lesions presents an extremely challenging problem. Stem cells (SC) extracted from certain tissues, such as adipose tissue, can differentiate into various cell types. Therefore, we have tried to use such cells to stimulate healing in a purulent environment. Methods: In the beginning, we designed a phase I clinical trial, involving five patients with Crohn’s disease. We inoculated nine fistulas in four patients with autologous adipose-derived stem cells (ADSC) and were followed at least 8 weeks. Seventy-five percent became healed, and 25% showed a decrease in output flow. No adverse effects were observed in any patient. This study evidenced that such cells are safe. Then, we started a research line using SC in different suppurative environments. During the course of these studies, we had the opportunity to treat a patient with perianal hidradenitis suppurativa using our current protocol of ADSC transplantation. Eight weeks after injection, patient had no perianal suppuration, and a year later remains well. Discussion: The biological mechanism that underlies the therapeutic success of ADSC transplantation is unknown. Cell differentiation, secretion of growth factors or immunomodulatory effects have been suggested. No ethical conflicts were identified by our Ethics Committee, because the cells were autologous. Conclusions: Our study shows that ADSC are safe for the treatment of suppurative processes. The actual number of patients included and the uncontrolled nature of these pilot studies do not allow demonstration of the effectiveness of the treatment. However, the results encourage the performance of further studies. Acknowledgements The HSF would like to thank the Dessau Medical Center for co-hosting the event, and the Task Force ‘‘Acne and Rosacea’’ of the European Academy of Dermatology and Venerology (EADV) for co-organizing the event. ‘‘Directions 2006’’ was also supported in part by unrestricted gifts from Creative Fusion, Inc., DVV Stadtwerke Dessau, Abbott Immunology, Labcatel Dermatology, and by private donations. The HSF would like to extend special thanks to Mayor Otto and the City of Dessau, the Departments of Dermatology and Immunology at the Dessau Medical Center, the helpful staff at the Steigenberger Hotel, and the residents of Dessau for their hospitality and support. Donations to the Hidradenitis Suppurativa Foundation, Inc. (HSF), are strongly encouraged and warmly welcomed. Gifts to the HSF will help promote innovative basic and clinical research into the as yet obscure cause(s) and into the clinically unsatisfactory management of Hidradenitis Suppurativa, which affects about 1% of the population worldwide. The HSF is a non-profit organization registered in the state of California, USA. For more information, please visit http://www. hs-foundation.org. San Diego, CA, April 2006 Hidradentitis Suppurativa Foundation, Inc. Michelle Barlow, President Rob Howes, Vice President Ralf Paus, Director of Basic Research Christos C. Zouboulis, Director of Clinical Research HSF Abstracts 482

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